Packard Children’s surgeons repair deformities of face, neck, skull

9:44 February 8, 2010

Lucile Packard Children’s Hospital’s craniofacial and plastic surgery team is up to the challenge. This group of clinicians and researchers provides innovative, multidisciplinary expertise on congenital defects, developmental problems and traumatic injuries affecting the head.

“In one day, we can have patients see all of the specialists in the craniofacial clinic,” said H. Peter Lorenz, MD, director of craniofacial surgery and service chief for plastic surgery at Packard Children’s. The clinic provides one-stop access to practitioners from craniofacial surgery, neurosurgery, otolaryngology, orthodontics, genetics, psychiatry, audiology, speech-language therapy, ophthalmology, nursing and social work. “Families like that—it’s a long half-day for them, but they don’t have to make a lot of separate doctor trips,” Lorenz said.

After clinic, the team writes a coordinated care plan for each child, ensuring all treatments will be delivered at the right stage of development. Kids with congenital defects such as cleft palate or malformed facial or skull bones need medical attention in infancy; others start treatment later in childhood or adolescence. “Any patient born with a clear deformity of the face should be referred to us right after birth,” said craniofacial surgeon Rohit Khosla, MD.

Cleft lip and palate, hemifacial microsomia—in which one side of the face is smaller than the other—and craniosynostosis, or prenatal fusion of the skull plates, can be most successfully repaired with early treatment. “We can stretch bone to make it grow,” Khosla said.

Packard Children’s research has produced pioneering advances in bone distraction, in which a surgeon cuts the bone in two and braces the two halves a few millimeters apart. The surgeon widens the gap a little each day until the bone is large enough, and the body fills the space with new bone.

The team uses distraction to treat undersized jawbones, which cause a too-small mouth that crowds the tongue back into the airway. Distraction is also appropriate for facial asymmetry caused by underdevelopment of one side of the face. After the two- to three-month procedure ends, the enlarged bones grow at the same pace as other bones in the face; many children need no further surgery.

Cleft lip and palate patients, on the other hand, typically require a series of surgeries. “As they grow they need little alterations,” Khosla said. When a patient is 3 months of age, a surgeon performs the first operation to close the gap in the lip. The palate can be closed at about 9 months, and follow-up surgeries on the gum line and nose occur at age 7 and in the teen years, respectively. Some kids need additional reconstruction to facilitate speech or enlarge the upper jaw.

Craniosynostosis treatment brings craniofacial and plastic surgeons together with Packard Children’s neurosurgery team to correct prenatal fusion of the skull bones, a defect that restricts brain growth. The team opens the seams in the skull so the brain will have room to develop, and the infant’s body grows new bone to fill the gap. “That ability is lost by age 2,” Lorenz said, “so we have a window when we can do these operations.”

Many patients come to Packard Children’s for evaluation and repair of soft-tissue defects of the face, head and neck. The team offers facial reconstruction for trauma patients, reanimation surgery for kids who lack nerves and muscles used to smile, and treatment of varied skin, vascular and cosmetic problems.

“We’re working with children to really change their lives,” said Khosla. “That’s the most rewarding part of what I do.”

Khosla and James Chang, MD, who chairs the division of plastic surgery at Stanford Hospital & Clinics, restore smiles to children who lack facial nerve and muscle function. By transplanting a small muscle from the thigh to the face, they give back a child’s ability to show facial expressions. Similarly, during microsurgery for traumatic wounds, they transplant tissue from other parts of the body to the wounded area and meticulously connect blood vessels and nerves so the transplant can thrive.

Packard Children’s plastic surgeons also have substantial expertise in managing vascular malformations such as hemangiomas. These non-malignant blood vessel tumors cause red or purple blotches on the skin of about 12 percent of infants. Though hemangiomas often shrink, they sometimes leave large scars behind. If patients are referred early, the team can help families decide whether surgery is necessary. Removing other skin malformations, including large nevi (moles), is also a routine part of the team’s practice. They frequently implant balloon expanders under the skin to generate new tissue for repairs, minimizing later scarring.

Lorenz hopes to one day do his job without leaving any scars at all. That’s why his lab is studying how fetal skin repairs itself. The lab’s 2008 discovery of Dot cells, key players in the fetal ability to heal without scarring, sparked excitement for the future of scarless surgery.

“Dot cells are like soldiers for stem cells,” Lorenz explained. Unlike stem cells, they don’t become new tissue in a test tube; instead they home in on a wound and help make healthy new skin. Fetal mice have lots of Dot cells, whereas juvenile and adult mice have only a few. The same pattern holds for humans.

Lorenz hopes to one day be able to infuse Dot cells into children having skin surgery to prevent scars from forming. His research has already shown the strategy works in mice. “Dot cells know how to go to the wound site without any coaching,” he said. And the cells don’t appear to trigger immune rejection when swapped between animals. “In theory, our plan would be to culture Dot cells and have a supply available for use where you don’t have to worry about the donor,” he added. “We could inject the cells into patients with skin problems to enable scarless skin repair. The possibilities are really exciting.”

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